Journal of Yeungnam Medical Science

Case report

Superficial malignant peripheral nerve sheath tumor from recurrent neurofibroma in the abdominal wall of a patient without neurofibromatosis type 1: Chang Yeon Jung, Jung Min Bae, Joon Hyuk Choi, Ki Hoon Jung; Yeungnam Univ J Med. 2019;36(1):63-66. Published online December 20, 2018; DOI: https://doi.org/10.12701/yujm.2019.00031

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Malignant peripheral nerve sheath tumor (MPNST) is rare, accounting for 5–10% of all soft tissue sarcomas. MPNST is characteristically aggressive and has a poor prognosis. Fifty percent of patients with MPNST have neurofibromatosis type 1 (NF1). NF-associated MPNST (NF-MPNST) occurs more often at younger ages than sporadic MPNST (sMPNST), but the survival difference is controversial. Superficial MPNST from a recurrent neurofibroma is extremely rare and only a limited number of cases have been reported in the literature. Herein, we report an unusual case of superficial MPNST from a recurrent neurofibroma in a patient without NF1.

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Sporadic neurofibroma of transverse colon in a patient without neurofibromatosis type 1: A case report
Toru Imagami, Saburo Sugita, Takaya Nagasaki, Masahiro Kimura, Keisuke Ito, Shingo Inaguma
International Journal of Surgery Case Reports.2020; 71: 19. CrossRef

Case Report

A Case of Type 1 Neurofibromatosis Associated with Multiple Metastatic Gastrointestinal Stromal Tumors.: Hyo Jin Jang, Sung Ae Koh, Da Eun Jeong, Ji Yoon Jung, Eun Ju Goo, Kyoung Hee Lee, Joon Hyuk Choi, Myung Soo Hyun; Yeungnam Univ J Med. 2013;30(2):105-108. Published online December 31, 2013; DOI: https://doi.org/10.12701/yujm.2013.30.2.105

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Abstract PDF: Type 1 neurofibromatosis (von Recklinghausen's disease, NF-1) is an autosomal-dominant neurocutaneous-disorder characterized by systemic cafe'-au-lait spots, multiple cutaneous neurofibromas, axillary or inguinal freckling, and Lisch nodules (pigmented iris hamartomas). Approximately 10-25% of NF1 patients have gastrointestinal neoplasms. Gastrointestinal stromal tumor (GIST) in patients with neurofibromatosis is most commonly found in the small bowel and the stomach, and approximately 60% of such patients have multiple tumors or multiple tumor sites. Although, the increased incidence of GIST in patients with neurofibromatosis is well documented in pathology literature in English, but has rarely been documented in Korea. Here, we report a case of multiple GISTs in a 48-year-old woman accompanied by NF1. She was admitted to Yeungnam University Hospital with complaints of melena and dyspnea. A contrast-enhanced computed tomography (CT) scan revealed that multiple soft tissue masses were occupying the entire peritoneal cavity. An ultrasonogram- guided biopsy was performed and the tumors were found to have been composed of tumor cells that were positive for c-kit protein. The patient was put on Imatinib mesylate treatment, and further follow-up will be carried out.

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